Know the basics

What is biliary atresia?

Biliary atresia is a rare condition that causes a blockage in the bile duct. Bile ducts in the liver, also called hepatic ducts, are tubes that carry bile from the liver to the gallbladder for storage and to the small intestine for use in digestion. Bile is a fluid made by the liver that serves two main functions: carrying toxins and waste products out of the body and helping the body digest fats and absorb the fat-soluble vitamins A, D, E, and K. When the bile duct is blocked, bile builds up in the liver and causes liver damage. This makes it hard for the liver to remove toxins from the body.

The two types of biliary atresia are fetal and perinatal. Fetal biliary atresia appears while the baby is in the womb. Perinatal biliary atresia is much more common and does not become evident until 2 to 4 weeks after birth. Some infants, particularly those with the fetal form, also have birth defects in the heart, spleen, or intestines.

How common is biliary atresia?

Biliary atresia is rare and only affects about one out of every 18,000 infants. The disease is more common in females, premature babies, and children of Asian or African American heritage.

Know the symptoms

What are the symptoms of biliary atresia?

The first symptoms of biliary atresia jaundice and yellow eyes. Jaundice can be hard to detect. Typically, babies born mild jaundice in the first 1-2 weeks and disappear from 2 to 3 weeks. However, in children with biliary obstruction, jaundice will increasingly progressive.

There are also other symptoms of biliary obstruction, such as:

Dark urine;

Separate gray or white;

Growth is slow.

There may be other symptoms and signs are not mentioned. If you have any questions about these signs, please consult your doctor.

When should I see my doctor?

If 2 to 3 weeks after birth, your child still has symptoms such as jaundice or white and gray stools. You should take her to the doctor immediately.

Know the causes

What causes biliary atresia?

Know the risk factors

What increases my risk for biliary atresia?

There are some factors that can increase your risk for biliary atresia, which may include:

Having a viral or bacterial infection after birth;

Having an autoimmune disorder that is attacking the liver or bile ducts;

Having a genetic mutation;

Congenital defects of the liver and bile ducts;

And exposure to hazardous substances.

Understand the diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is biliary atresia diagnosed?

To determine a proper diagnosis, your doctor may perform the following tests:

Medical history and physical examination;

Blood tests;

Abdominal x-rays;

Ultrasound;

Liver scans;

And/or liver biopsy.

How is biliary atresia treated?

Biliary atresia is usually treated with surgery, specifically called the Kasai procedure, or liver transplant.

Kasai procedure

This is usually the first treatment for biliary atresia. During the Kasai procedure, the surgeon removes the blocked bile ducts in infants and brings the loop of intestine up to replace them. Then the bile will flow straight to the small intestine. Cases of successful surgery, patients are in good health and not experiencing liver problems.

If the Kasai operation fails, the child will need a liver transplant within 1-2 years. Even after the successful transplant, most children will be at risk for obstructive biliary cirrhosis in adulthood. Therefore, children need to be re-examined regularly to monitor the activity of the liver.

Liver transplant

The progress and advances in transplant surgery have increased the availability and efficient use of livers for transplants in children. Before, only liver transplants were performed when the liver was available from a matched doner, who were deceased small children. Now with new advancements in surgery, surgeons can use adult livers for liver transplants in children. This is called reduced size or split-liver transplant

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage biliary atresia?

The following lifestyles and home remedies might help you cope with biliary atresia:

Infants with biliary atresia usually lacking many nutrients and need special diets, as they grow older. Therefore, children need more calories in the daily diet;

After a liver transplant, most children may be able to eat normally. However, should always consult with your pediatrician what is the right diet for your child.
If you have any questions, please consult with your doctor to better understand the best solution for you.